P82: A NATIONAL AUDIT OF HEREDITARY AND AQUIRED ANGIOEDEMA IN NEW ZEALAND

Hereditary Angioedema (HAE) is a rare potentially life threatening genetic condition, but access to effective therapies can reduce mortality and improve quality of life. Patients with this condition in New Zealand remain uncharacterised by number, geographical distribution, severity or treatment experience. New Zealand Immunologists were invited to recruit patients with HAE and AAE to the audit or those identified as having Berinert® for hereditary or acquired angioedema. Participants were consented, their angioedema related health information was collected and they were invited to take part in telephone or face to face interviews about their experience of healthcare. Twenty-one patients with hereditary angioedema were recruited, three of whom had acquired angioedema C1 inhibitor deficiency. Three patients were diagnosed prior to the onset of symptoms due to the diagnosis of a family member with the disease by screening family members. The average diagnostic delay was 12.9 years. Variation in delay between different types of HAE, with the greatest mean delay being in Type 1 HAE at 18.5 years, 13 years in type 2 HAE, and 3.6 years in AAE. Within the cohort of 21 patients there were reports of 4 deaths of family members due to HAE. The majority of patients 19/21 (90%) had a written plan to present to the emergency department. Few (24%) had a Medic Alert® bracelet. In 2015 there were a total of 217 HAE attacks in 16 patients. Five patients (24%) were asymptomatic. Only one patient had angioedema of the upper airway in 2015 but did not require intubation. Six patients had 136 abdominal attacks; some with high frequency (range 1–52). Four patients said HAE had no impact on their life, 10 had minor impact, and 4 moderate and 3 described it as severe. This study characterises a cohort of AAE and HAE patients in New Zealand.